Osteogenesis Imperfecta Tarda (Case Report Tow cases) / 대한정형외과학회잡지

ABSTRACT

Two cases report on osteogenesis imperfecta tarda and review of literature has been made. The rare genetic disorder, osteogenesis imperfecta tarda is familiar to most orthopedic surgeons because of management of fracture and skeletal deformities, espicially bowing of long bone. Its clinical manifestations are known as bony fragility, blue sclera and deafness less frequently ligamentous laxity but its etiological factor is obscure; The basic defect is thought to involvement some abnormality of collagen. Recently the bony weakness caused by increased bony turnover rate. One case of osteogenesis imberrfecta tarda in 8 year old male was belonged to tarda type 1. (Falvo-classification), who could not walk due to marked anterolateral bowing of right tibia and the another to tarda type II. in 13 year old male with fresh fracture of right femoral shaft. The former was performed corrective multible osteotomy, realignment and intramedullary rod fixation for correction of bowing deformity of right tibia, and the later was managed the fracture of right femoral shaft with Russels traction and hip spica cast. The result of reported therapeutic mesure was good.